Article
Respiratory chain dysfunction and oxidative stress correlate with severity of primary CoQ10 deficiency.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 Jun 2008
Quinzii Catarina M, López Luis C, Von-Moltke Jakob, Naini Ali, Krishna Sindu, Schuelke Markus, Salviati Leonardo, Navas Plácido, DiMauro Salvatore, Hirano Michio
Abstract excerpt
Coenzyme Q(10) (CoQ(10)) is essential for electron transport in the mitochondrial respiratory chain and antioxidant defense. Last year, we reported the first mutations in CoQ(10) biosynthetic genes, COQ2, which encodes 4-parahydroxybenzoate: polyprenyl transferase; and PDSS2, which encodes subunit 2 of decaprenyl diphosphate synthase. However, the pathogenic mechanisms of primary CoQ(10) deficiency have not been...
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